Pheochromocytomas, MEN 2A and pregnancy: A case report with review of the literature

Pheochromocytoma in pregnancy is a rare condition with an estimated incidence of one in 54,000 pregnancies. Pheochromocytoma in the setting of multiple endocrine neoplasia (MEN) type 2A develops in approximately 40% of the patients with this germline mutation. MEN 2A patients with pheochromocytoma are often asymptomatic compared to those with nonsyndromic pheochromocytoma. We present a 28-year-old pregnant woman with a known MEN 2A mutation who was incidentally found to have a pheochromocytoma. She underwent an uncomplicated laparoscopic right adrenalectomy after proper α- and β-adrenergic blockade. Her serum fractionated metanephrines normalized prior to discharge from the hospital. The remainder of her pregnancy was uneventful and she delivered a health baby at term. Pheochromocytoma in pregnancy requires careful management to assure the well being of both the mother and the fetus. In pregnancy, laparoscopic surgical removal of the pheochromocytoma is feasible with excellent results when performed in the second trimester. In late pregnancy, medical management with close monitoring of the mother and fetus is recommended until fetal maturity is achieved. In the third trimester, cesarean section followed by open adrenalectomy is the preferred operation. An integrated and multidisciplinary approach with close communication among the endocrinologist, surgeon and perinatal services is paramount to achieve excellent outcomes for both patients. Women with MEN 2 should be advised to refrain from pregnancy until updated biochemical and imaging studies have ruled out pheochromocytoma.